Huang Laboratory

UCSF Department of Pathology

Pediatric Neuropathology and Developmental Neurobiology

Sun S, Ling SC, Qiu J, Albuquerque CP, Zhou Y, Tokunaga S, Li H, Qiu H, Bui A, Yeo GW, Huang, E.J., Eggan K, Zhou H, Fu XD, Lagier-Tourenne C, Cleveland DW. ALS-causative mutations in FUS/TLS confer gain and loss of function by altered association with SMN and U1-snRNP. Nat Commun 6:6171, 2015.
Berthet A, Margolis EB, Zhang J, Hsieh I, Zhang J, Hnasko TS, Ahmad J, Edwards RH, Sesaki H, Huang, E.J., Nakamura K. Loss of mitochondrial fission depletes axonal mitochondria in midbrain dopamine neurons. J Neurosci 34(43):14304-17, 2015.
Sephton CF, Tang AA, Kulkarni A, West J, Brooks M, Stubblefield JJ, Liu Y, Zhang MQ, Green CB, Huber KM, Huang, E.J., Herz J, Yu G. Activity-dependent FUS dysregulation disrupts synaptic homeostasis. Proc Natl Acad Sci U S A 111(44):E4769-78, 2014.
Tate MC, Lindquist RA, Nguyen T, Sanai N, Barkovich AJ, Huang, E.J., Rowitch DH, Alvarez-Buylla A. Postnatal growth of the human pons: a morphometric and immunohistochemical analysis. J Comp Neurol 523(3):449-62, 2014.
Fancy SP, Harrington EP, Baranzini SE, Silbereis JC, Shiow LR, Yuen TJ, Huang, E.J., Lomvardas S, Rowitch DH. Parallel states of pathological Wnt signaling in neonatal brain injury and colon cancer. Nat Neurosci (4):506-12, 2014.
Qiu H*, Lee S*, Shang Y, Wang WY, Au KF, Kamiya S, Barmada SJ, Finkbeiner S, Lui H, Tang AA, Oldham MC, Wang H, Shorter J, Filiano AJ, Roberson ED, Tourtellotte WG, Chen B, Tsai LH, Huang, E.J. ALS-associated mutation FUS-R521C causes DNA damage and RNA splicing defects. J Clin Invest.2014 Feb 10. [Epub ahead of print][Featured in JCI Scientific Show Stopper: http://www.jci.org/posts/132]
Wang, W.Y., Pan, L., Su, S.C., Quinn, E.J., Sasaki, M., Jimenez, J.C., Mackenzie, I.R., Huang, E.J. and Tsai, L.H. Interaction of FUS and HDAC1 regulates DNA damage response and repair in neurons. Nat Neurosci 16(10): 1383-91, 2013.
Heng, M.Y., Lin, S.T., Verret, L., Huang, Y., Kamiya, S., Padiath, Q.S., Tong, Y., Palop, J.J., Huang, E.J., Ptacek, L.J. and Fu, Y.H. Lamin B1 mediates cell-autonomous neuropathology in a leukodystrophy mouse model. J Clin Invest 123(6): 2719-29, 2013.
Tang, M., Luo, S.X., Tang, V. and Huang, E.J. Temporal and spatial requirements of smoothened in ventral midbrain neuronal development. Neural Dev 8(1): 8, 2013.
Cleaver, J.E., Bezrookove, V., Revet, I. and Huang, E.J. Conceptual developments in the cause of Cockyane syndrome. Mech Ageing Dev 134 (5-6): 284-90, 2013.
Shang, Y., Doan, C., Arnold, T.D., Lee, S., Tang, A.A., Reichardt, L.F. and Huang, E.J. Transcriptional Corepressors HIPK1 and HIPK2 Control Angiogenesis via TGF-β-TAK1-depdendent Mechanisms. PLoS Biology 11(4):e1001527, 2013.
Filiano, A.J., Martens, L.H., Young, A.H., Warmus, B.A., Zhou, P., Diaz-Ramirez, G., Jiao, J., Zhang, Z., Huang, E.J., Gao, F.B., Farese, Jr. R.V. and Roberson, E.D. Dissociation of frontotemporal dementia-related deficits and neuroinflammation in Progranulin haploinsufficient mice. J Neurosci 33(12): 5352-5361, 2013.
Martens, L.H., Zhang, J., Zhou, P., Kamiya, S., Sun, B., Min, S.-W., Zhang, Y., Diaz-Ramirez, G., Gan, L., Huang, E.J.* and Farese, Jr., R.V.* Progranulin deficiency promotes microglial activation, proinflammtory cytokine production, and aggravated neuronal loss in toxin-induced neurodegeneration. J Clin Invest 122(11): 3955-9, 2012 (*co-corresponding authors)
Nobuta, H., Ghiani, C.A., Paez, P.M., Spreuer, V., Dong, H., Korsak, R.A., Manukyan, A., Li, J., Vinters, H.V., Huang, E.J., Rowitch, D.H., Sofroniew, M.V., Campagnoni, A.T., de Vellis, J. and Waschek, J.A. STAT3-Mediated astrogliosis protects myelin development in neonatal brain injury. Ann Neurol. 72(5): 750-65, 2012.
Kwan, K.Y., Lam, M.M.S., Johnson, M.B., Dube, U., Shim, S., Rasin, M-R., Sousa, A.M.M., Fertuzinhos, S., Chen, J.G., Arellano, J.I., Chan, D.W., Pletikos, M., Vasung, L., Rowitch, D.H., Huang, E.J., Schwartz, M.L., Willemsen, R., Oostra, B.A., Rakic, P., Heffer, M., Kostovic, I., Judas, M. and Sestan, N. Species-Dependent Posttranscriptional Regulation of NOS1 by FMRP in the Developing Cerebral Cortex. Cell 149, 899Ð911, 2012.
Revet, I., Feeney, L., Tang, A.A., Huang, E.J. and Cleaver, J.E. Dysmyelination not demyelination causes neurological symptoms in preweaned mice in a murine model of Cockayne syndrome. Proc Natl Acad Sci (USA) 109(12): 4627-32, 2012.
Arnold, T.D., Ferrero, G.M., Qiu, H., Phan, I.T., Akhurst, R.J., Huang, E.J. and Reichardt, L.F. Defective Retinal Vascular Endothelial Cell Development As a Consequence of Impaired Integrin αVβ8-Mediated Activation of Transforming Growth Factor-β. J Neurosci 32(4): 1197-206, 2012.
Mao, J.H., Wu, D., Kim, I.J., Kang, H.C., Wei, G., Climent, J., Kumar, A., Pelorosso, F.G., Delrosario, R., Huang, E.J. and Balmain, A. Hipk2 cooperates with p53 to suppress ?-ray radiation-induced mouse thymic lymphoma. Oncogene 31(9): 1176-80, 2012.
Sanai, N., Nguyen, T., Ihrie, R.A., Mirzadeh, Z., Tsai, H.H., Wong, M., Gupta, N., Berger, M.S., Huang, E.J., Garcia-Verdugo, J.M., Rowitch, D.H. and Alvarez-Buylla, A. Corridors of Migratory Neurons in Human Brain and their Decline During Infancy. Nature 478(7369): 382-6, 2011.
Chalazonitis, A., Tang, A.A., Shang, Y., Pham, T.D., Hsieh, I., Setlik, W., Gershon, M.D. and Huang, E.J. Homeodomain Interacting Protein Kinase 2 Regulates Postnatal Development of Enteric Dopamine Neurons and Glia via BMP Signaling. J Neurosci 31(39): 13746-57, 2011.
Fancy, S.P.J., Harrington, E.P., Baranzini, S.E., Zhao, C., Silberesis, J.C., Otero, J.J., Huang, E.J., Nusse, R., Franklin, R.J.M. and Rowitch, D.H. Wnt inhibitor AXIN2 as regenerative medicine target in multiple sclerosis and neonatal white matter injury. Nature Neurosci 14(8): 1009-16, 2011.
Lee, C., Hu, T., Nguyen, J.M., Zhang, J., Huang, E.J. and Chan, J.Y. Loss of Nrf1 function in the brain causes age-dependent neurodegeneration. Proc Natl Acad Sci (USA) 108(20): 8408-13, 2011.
Silbereis, J.C., Huang, E.J., Back, S.A., Rowitch, D.H. Towards improved animal models of neonatal white matter injury associated with cerebral palsy. Dis Model Mech. 3:678-88, 2010. (Review)
Reyes, N.A., Fisher, J.K., Austgen, K., VandenBerg, S., Huang, E.J. and Oakes, S.A. Blocking the mitochondrial apoptotic pathway protects against neurodegeneration. J Clin Invest 120: 3673-9, 2010.
Tang, M., Villaescusa, J.C., Luo, S.X., Guitarte, C., Lei, S., Miyamoto, Y., Maketo, M.M., Arenas, E. and Huang, E.J. Interactions of Wnt/b-catenin signaling and sonic hedgehog regulate the neurogenesis of midbrain dopamine neurons. J Neurosci 30: 9280-9, 2010.
Huang, E.J., Zhang, J., Geser, F., Trojanowski, J.Q., Strober, J.B., Dickson, D.W., Brown, R.H., Jr., Shapiro, B.E. and Lomen-Hoerth, C. Extensive FUS-immunoreactive Pathology in Juvenile amyotrophic lateral sclerosis with Basophilic Inclusions. Brain Pathology 20: 1069-76, 2010.
Tang, M., Miyamoto, Y. and Huang, E.J. Multiple roles of b-catenin in controlling the neurogenic niche for midbrain dopamine neurons. Development 136: 2027-2038, 2009.
Wei, G., Ku, S., Saito, S., Tang, A.A., Mao, J.H., Appella, E., Balmain, A. and Huang, E.J. HIPK2 represses b-catenin-mediated transcription, epidermal stem cell expansion and skin tumorigenesis. Proc Natl Acad Sci (USA) 104: 13040-13045, 2007.
Laposa, R., Huang, E.J. and Cleaver, J.E. Increased apoptosis, p53 upregulation, and cerebellar neuronal degeneration in repair deficient Cockyane Syndrome mice. Proc Natl Acad Sci (USA) 104: 1389-1394, 2007.
Zhang, J., Pho, V., Bonasera, S.J., Holzmann, J., Helmuth, J., Tang, A.A., Janak, P.H., Tecott, L.H. and Huang, E.J. Essential function of HIPK2 in TGFb-dependent survival of midbrain dopamine neurons. Nature Neuroscience 10:77-86, 2007.
Zhang, J. and Huang, E.J. Dynamic Expression of Neurotrophic Factor Receptors in Postnatal Spinal Motoneurons and Mouse Model of ALS. J Neurobiol 66, 882-895, 2006.
Huang, E.J., Li, H., Tang, A.A., Wiggins, A.K., Neve, R.L., Zhong, W., Jan, L.Y. and Jan, Y.N. Targeted deletion of Numb and Numblike reveals their essential functions in axon arborization. Genes & Dev 19(1): 138-151, 2005.
Doxakis, E., Huang, E.J. and Davies, A.M. Homeodomain interacting protein kinase 2 (HIPK2) regulates apoptosis in developing sensory and sympathetic neurons. Curr Biol 14(19): 1761-1765, 2004.
Wiggins, A.K., Wei, G., Doxakis, E., Wong, C., Tang, A.A., Zeng, K., Luo, E.J., Neve, R.L., Reichardt, L.F. and Huang, E.J. Interaction of Brn3a and HIPK2 mediates transcriptional repression of sensory neuron survival. J Cell Biol 167 (2): 257-267, 2004.
Huang, E.J. and Reichardt, L.F. Trk receptors: Roles in Neuronal Signal Transduction. Ann Rev Biochem 72:609-642, 2003. (Review)
Huang, E.J. and Reichardt, L.F. Neurotrophins: Roles in Neuronal Development and Function. Ann Rev Neurosci 24:677-736, 2001. (Review)
Huang, E.J., Liu, W., Fritzsch, B., Bianchi, L.M., Reichardt, L.F. and Xiang, M. Brn-3a is a transcriptional regulator of soma size, target field innervation and axon pathfinding of inner ear sensory neurons. Development 128, 2421-2432, 2001.
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